case report ct and mr unilateral brain features secondary...

Case ReportCT and MR Unilateral Brain Features Secondary to NonketoticHyperglycemia Presenting as Hemichorea-Hemiballism

Víctor Manuel Suárez-Vega,1 Carlos Sánchez Almaraz,1 Ana Isabel Bernardo,1

Ricardo Rodríguez-Díaz,1 Ana Díez Barrio,2 and Leticia Martín Gil2

1Radiology Department, Hospital Infanta Elena, Valdemoro, 28342 Madrid, Spain2Neurology Department, Hospital Infanta Elena, Valdemoro, 28342 Madrid, Spain

Correspondence should be addressed to Vıctor Manuel Suarez-Vega; [email protected]

Received 16 November 2015; Revised 10 April 2016; Accepted 20 April 2016

Academic Editor: Atsushi Komemushi

Copyright © 2016 Vıctor Manuel Suarez-Vega et al. This is an open access article distributed under the Creative CommonsAttribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work isproperly cited.

Hemichorea-hemiballism is an unusual hyperkineticmovement disorder characterized by continuous involuntarymovements of anentire limb or both limbs on one side of the body.The acute onset of this disorder occurs with an insult in contralateral basal ganglia.Ischemic events represent the most common cause. Nonketotic hyperglycemia comes in second place. Nonketotic hyperglycemichemichorea-hemiballism (NHH) is a rare cause of unilateral brain abnormalities on imaging studies confined to basal ganglia(mainly putaminal region as well as caudate nucleus). Subtle hyperdensity in striatal region can be found on CT studies whereasbrain MR imaging typically shows T1 hyperintensity and T2 hypointensity in the basal ganglia contralateral to the movements.Diagnosis is based on both glucose levels and neuroimaging findings. Elevated blood glucose and hemoglobin A1c levels occurwith poorly controlled diabetes. In this case report, our aim is to present neuroimaging CT andMR unilateral findings in an elderlywoman secondary to nonketotic hyperglycemia presenting as hemichorea-hemiballism.

1. Introduction

Hemichorea-hemiballism is an unusual hyperkinetic move-ment disorder characterized by continuous involuntarymovements of an entire limb or both limbs on one side ofthe body. These movements are mostly irregular, variable inamplitude, and with no recognizable pattern [1].

Bedwell first described hemichorea-hemiballism associ-ated with hyperglycemia in 1960. He reported the case of a 57-year-old woman with hemiballism that resolved as the bloodglucose levels were normalized [2].

It was Yahikozawa et al. who first reported 3 diabeticpatients with the unique combination of hemiballism andstriatal hyperintensity on T1-weighted MRI as a new syn-drome in 1994 [3].

NHH is most frequently reported in elderly patients,typically Asian, who have type II diabetes. The majority ofcases published in the literature involve female patients [4].

Although this syndrome usually occurs in patients withpreviously known diabetes, it could also be an initial mani-festation of diabetes [5].

2. Case Presentation

A 57-year-old female patient from East Europe was admittedin the emergency roomof our institutionwith chest pain. Herserum glucose and hemoglobin A1c levels were 800mg/dLand 15.9%, respectively. There were no ketones in the serum.Serum glucose was treated by insulin continuous perfusionand the patient was discharged.

A few hours later, she was readmitted presenting involun-tary and violent movements on the left side of the body.

An unenhanced cranial CT was performed to rule outan ischemic event. Findings were initially misdiagnosed,reporting hypoattenuation of left basal ganglia instead ofconsidering an abnormal increased attenuation on right basal

Hindawi Publishing CorporationCase Reports in RadiologyVolume 2016, Article ID 5727138, 4 pageshttp://dx.doi.org/10.1155/2016/5727138

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Figure 1: Findings: unenhanced axial brain CT depicts hyperdensestriatum (caudate nucleus and putamen). This finding is better seenusing narrow window width and higher center settings.

ganglia. Using narrow window width and slightly highercenter settings better depicted this finding (Figure 1).

As correction of hyperglycemia did not lead to improve-ment of the involuntarymovements, the patient was admittedin the Intensive CareUnit and an aggressive intravenous ther-apy was performed. Initially, risperidone up to 2mg/dL and acontinuous perfusion of clonazepamwas administrated, withno response. Further treatment with tetrabenazine 25mgtwice a day was necessary for improvement of themovementsin the next few days.

MR imaging of the brain was performed 2 days after thesecond admission showing right basal ganglia hyperintensityon T1-weighted sequences and fairly normal signal intensityon T2-weighted and FLAIR sequences (Figure 2). The appar-ent diffusion coefficients (ADCs) were slightly lower in rightbasal ganglia compared to those on the left side. ADC valueswere symmetrical on both basal ganglia (Figure 3).

One month later—movement disorders completelyrecovered—the patient was reimagined, depicting a strikingincrease on T1-weighted signal intensity, whereas the signalintensity of the rest of the sequences was within normallimits (Figure 4).

Both MR studies performed with one month differencedid not show any abnormality within right striatum onT2 gradient echo sequences (Figure 5). However, a 10-month follow-upMR scan showed hypointensity within rightstriatum on susceptibility weighted images (SWI) (Figure 6).

Several follow-up MR studies have been performed withan elapsed time of 4 months and 10 months. Whereas the4-month follow-up study still depicted right basal gangliahyperintensity, the 10-month MR study finally confirmed atotal regression of signal abnormalities, showing a normalappearance right striatum. No signs of striatum atrophy wereshown (Figure 7).

3. Discussion

The underlying pathophysiology of imaging changes des-cribed in NHH is poorly understood.

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Figure 2: Axial T1-weighted sequence depicts right basal gangliahyperintensity with unremarkable signal abnormality on axial T2-weighted.

Some mechanisms include blood hyperviscosity causedby hyperglycemia, leading to blood brain barrier disrup-tion; increased sensitivity of dopaminergic receptors inpostmenopausal period, remember female predominance;decreased gamma-aminobutyric acid (GABA) in striatumsecondary to a nonketotic state.

In addition, acute infarct, petechial hemorrhage, myeli-nolysis, and calcium deposition have also been postulated[6, 7]. MR findings suggest that petechial hemorrhage is lessplausible since if the high signal on T1 corresponds withmethemoglobin it should also be high signal on T2. Thisis not reported in our case or in previous cases [7]. Wecould not find any alterations on T2 gradient echo imagesinvolving right striatum. However, the 10-month follow-upscan showed slightly hypointense signal within right striatumon SW images. To our knowledge, there is only one seriesof three cases describing SWI findings in NHH syndrome[8].These authors explain T1 hyperintensity from the proteinhydration layer inside the cytoplasm of swollen gemistocytesappearing after an acute cerebral injury.These astrocytes alsoexpress metallothionein with zinc, which is thought to be thecause of asymmetric hypointensity of the posterior putamenon SWI.

Other authors suggest that high T1 signal could be relatedto manganese accumulation on gemistocytes [9].

The differential diagnosis of conditions presentingwith hemichorea includes ischemic or hemorrhagic stroke,vasculitis, central nervous system lupus, mass lesions,

Case Reports in Radiology 3

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Figure 3: ADC map shows slight decreased apparent diffusioncoefficient in right basal ganglia. Symmetrical normal ADC valuesone month later.

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Figure 4: Striking increase of T1-weighted signal intensity withinright striatum one month after the onset of symptoms. Patient wasasymptomatic when this MR study was performed.

multiple sclerosis, thyrotoxicosis, and drugs (such as neuro-leptics or levodopa) [10].

However, clinical history and laboratory tests with strik-ing increase of glucose levels make all these diagnosesunlikely.

Other causes to increased T1 signal in the basal gangliaare hypertensive hemorrhages, basal ganglia calcifications,Tay-Sachs disease, and tuberous sclerosis. A lenticulostriateisolated ischemic stroke can lead to a mismatch effect of falsehigh signal on putaminal region.

Other metabolic or toxic disorders showing T1 signalincrease include chronic hepatic encephalopathy, the most

Figure 5: Axial T2 gradient echo sequence shows no signal abnor-mality within right striatum but some scattered blooming artifactswithin left thalami consistent with petechial hemorrhages.

Figure 6: Axial minimum intensity projection of susceptibil-ity weighted imaging at 10-month follow-up depicts slightlyhypointense signal within right striatum.

frequent, manganese toxicity during long-term parenteralnutrition, postcardiac arrest encephalopathy, hypoglycemiccoma, hypothyroidism, neurofibromatosis, Fahr disease,Wil-son disease, and carbon monoxide poisoning. In all theseconditions a bilateral and symmetrical involvement is usuallypresent [11].

Clinical course in patients with NHH is usually favorableand symptoms tend to resolve spontaneously within nor-malization of glucose levels. In addition, striatal high signalintensitymay show resolutionwithin a variable period of 2–11months. In our case, T1 findings showed not only persistencybut also increase of the signal whereas the other sequencespartially resolved.Wemay emphasize that a period of 1monthseparated both studies. Further follow-up studies confirmedresolution of findings in a period of 10 months, as reportedpreviously in the literature.

One possible explanation could be acute putaminaldysfunction, secondary to hyperglycemic or hyperosmolarinsult. This functional alteration could be associated with

4 Case Reports in Radiology

Figure 7: 4-month and 10-month follow-up MR studies. SagittalT1 depicts persistent hyperintensity within right striatum (greenarrow), whereas a 10-month follow-up MR shows complete regres-sion of signal abnormalities.

some degree of Wallerian degeneration of the internal whitematter of the putamen. Protein desiccation occurring in thecourse ofWallerian degeneration could explain theCThyper-attenuation and theMRandDWimaging patterns in the earlyphase, as well as the variable evolution of imaging featureswith time [12]. In our patient, no signs of atrophy affectingright striatum were found in follow-up studies after thedisappearance of high intensity signals on T1 sequences. Thismight be explained by the early improvement in hemichorea-hemiballism, as suggested by the meta-analysis of Oh et al.[4].

In addition, we were not able to find an explanation forthe increased signal on T1 sequences after one month onset.Some studies with larger number of cases report an increasedT1 signal on follow-up MR scans compared to baseline CTimages, with a posterior resolution of MR abnormalities [11].

4. Conclusion

NHH is a unique clinical-radiological entity with peculiar CTand MRI findings.

An early recognition is mandatory in order to establishthe correct therapy (normalization of glucose levels) andavoid unnecessary treatments.

Competing Interests

The authors declare that there are no competing interestsregarding the publication of this paper.

References

[1] R. B. Postuma and A. E. Lang, “Hemiballism: revisiting a classicdisorder,”TheLancet Neurology, vol. 2, no. 11, pp. 661–668, 2003.

[2] S. F. Bedwell, “Some observations on hemiballismus,” Neurol-ogy, vol. 10, no. 6, pp. 619–622, 1960.

[3] H. Yahikozawa, N. Hanyu, K. Yamamoto et al., “Hemiballismwith striatal hyperintensity on T1-weighted MRI in diabeticpatients: a unique syndrome,” Journal of the Neurological Sci-ences, vol. 124, no. 2, pp. 208–214, 1994.

[4] S.-H. Oh, K.-Y. Lee, J.-H. Im, andM.-S. Lee, “Chorea associatedwith non-ketotic hyperglycemia and hyperintensity basal gan-glia lesion on T1-weighted brainMRI studyameta-analysis of 53cases including four present cases,” Journal of the NeurologicalSciences, vol. 200, no. 1-2, pp. 57–62, 2002.

[5] J. J. Lin, G. Y. Lin, C. Shih, and W. C. Shen, “Presentationof striatal hyperintensity on T1-weighted MRI in patientswith hemiballism-hemichorea caused by non-ketotic hyper-glycemia: report of seven new cases and a review of literature,”Journal of Neurology, vol. 248, no. 9, pp. 750–755, 2001.

[6] S. Ohara, S. Nakagawa, K. Tabata, and T. Hashimoto, “Hemibal-lismwith hyperglycemia and striatal T1-MRI hyperintensity: anautopsy report,”Movement Disorders, vol. 16, no. 3, pp. 521–525,2001.

[7] D.-E. Shan, D. M. T. Ho, C. Chang, H.-C. Pan, and M. M. H.Teng, “Hemichorea-hemiballism: an explanation for MR signalchanges,” American Journal of Neuroradiology, vol. 19, no. 5, pp.863–870, 1998.

[8] A. Cherian, B. Thomas, N. N. Baheti, T. Chemmanam, andC. Kesavadas, “Concepts and controversies in nonketotichyperglycemia-induced hemichorea: further evidence fromsusceptibility-weighted MR imaging,” Journal of Magnetic Res-onance Imaging, vol. 29, no. 3, pp. 699–703, 2009.

[9] M. Fujioka, T. Taoka, Y. Matsuo et al., “Magnetic resonanceimaging shows delayed ischemic striatal neurodegeneration,”Annals of Neurology, vol. 54, no. 6, pp. 732–747, 2003.

[10] J. S. Hawley andW. J.Weiner, “Hemiballismus: current conceptsand review,” Parkinsonism and Related Disorders, vol. 18, no. 2,pp. 125–129, 2012.

[11] P.-H. Lai, R. D. Tien, M.-H. Chang et al., “Chorea-ballismuswith nonketotic hyperglycemia in primary diabetes mellitus,”American Journal of Neuroradiology, vol. 17, no. 6, pp. 1057–1064,1996.

[12] M.Wintermark, N. J. Fischbein, P.Mukherjee, E. L. Yuh, andW.P. Dillon, “Unilateral putaminal CT, MR, and diffusion abnor-malities secondary to nonketotic hyperglycemia in the settingof acute neurologic symptoms mimicking stroke,” AmericanJournal of Neuroradiology, vol. 25, no. 6, pp. 975–976, 2004.

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